BMJ Open (Jul 2023)

Preoperative versus postoperative survival in patients with univentricular heart: a nationwide, retrospective study of patients born in 1990–2015

  • Gunnar Erikssen,
  • Jamil Aboulhosn,
  • Knut Liestøl,
  • Mette E Estensen,
  • Ola Gjesdal,
  • Gaute Døhlen,
  • Harald Lauritz Lindberg,
  • Gunnar Wik,
  • Henrik Holmstrøm,
  • Tom Nilsen Hoel,
  • Sigurd Birkeland,
  • Erik Thaulow,
  • Kjell Johan Saatvedt,
  • Egil Seem

DOI
https://doi.org/10.1136/bmjopen-2022-069531
Journal volume & issue
Vol. 13, no. 7

Abstract

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Objectives Few data exist on mortality among patients with univentricular heart (UVH) before surgery. Our aim was to explore the results of intention to perform surgery by estimating preoperative vs postoperative survival in different UVH subgroups.Design Retrospective.Setting Tertiary centre for congenital cardiology and congenital heart surgery.Participants All 595 Norwegian children with UVH born alive from 1990 to 2015, followed until 31 December 2020.Results One quarter (151/595; 25.4%) were not operated. Among these, only two survived, and 125/149 (83.9%) died within 1 month. Reasons for not operating were that surgery was not feasible in 31.1%, preoperative complications in 25.2%, general health issues in 23.2% and parental decision in 20.5%. In total, 327/595 (55.0%) died; 283/327 (86.5%) already died during the first 2 years of life. Preoperative survival varied widely among the UVH subgroups, ranging from 40/65 (61.5%) among patients with unbalanced atrioventricular septal defect to 39/42 (92.9%) among patients with double inlet left ventricle. Postoperative survival followed a similar pattern. Postoperative survival among patients with hypoplastic left heart syndrome (HLHS) improved significantly (5-year survival, 42.5% vs 75.3% among patients born in 1990–2002 vs 2003–2015; p<0.0001), but not among non-HLHS patients (65.7% vs 72.6%; p=0.22)—among whom several subgroups had a poor prognosis similar to HLHS. A total of 291/595 patients (48.9%) had Fontan surgeryConclusions Surgery was refrained in one quarter of the patients, among whom almost all died shortly after birth. Long-term prognosis was largely determined during the first 2 years. There was a strong concordance between preoperative and postoperative survival. HLHS survival was improved, but non-HLHS survival did not change significantly. This study demonstrates the complications and outcomes encountering newborns with UVH at all major stages of preoperative and operative treatment.